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Download Immunodeficiencies
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I. Primary immunodeficiencies â phagocytic cell defects Chédiak-Higashi syndrome ⢠Clinical features: recurrent, severe, pyogenic infections (streptococcal, staphylococcal) ⢠Defective intracellular killing of bacteria (neutrophils contain abnormal giant lysosomes HyperIgE (Jobâs) syndrome ⢠Mutation of STAT3 gene ⢠Recurrent âcoldâ staphylococcal abscesses, chronic eczema, otitis media ⢠Extremely high serum IgE levels ⢠Treatment: ATB LAD syndrome â Leukocyte Adhesion Deficiency (neutrophil adhesion molecules deficiency) ⢠LAD I â integrins expression deficiency ⢠LAD II â selectins expression deficiency
